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Haemangiomas and soft tissue and\/or skeletal hypertrophy were present in all 47 patients; venous varicosities developed in 37 (79 per cent). There was no clinical evidence of macrofistulous arteriovenous communications in any patient. Thromboembolic episodes occurred in five children (11 per cent) and 25 (53 per cent) experienced thrombophlebitis. The Kasabach-Merritt syndrome was observed in 21 (45 per cent) and six (13 per cent) presented with high-output cardiac failure. Other manifestations included haematuria in five (11 per cent), rectal or colonic haemorrhage in six (13 per cent), and vaginal, vulval or penile bleeding in six (13 per cent) children with visceral and pelvic haemangiomas. In 26 patients (55 per cent) symptomatic treatment only was required. Surgery was undertaken in selected cases for complications of the haemangioma, for cosmetic reasons and for chronic venous insufficiency. Only one of four children who underwent resection of varicose veins improved. There was no death, but significant morbidity was associated with the treatment of Kasabach-Merritt syndrome and high-output cardiac failure.<\/jats:p>","DOI":"10.1002\/bjs.1800820615","type":"journal-article","created":{"date-parts":[[2007,3,3]],"date-time":"2007-03-03T22:00:54Z","timestamp":1172959254000},"page":"757-761","source":"Crossref","is-referenced-by-count":137,"title":["Klippel-Trenaunay syndrome: Clinical features, complications and management in children"],"prefix":"10.1093","volume":"82","author":[{"given":"M","family":"Samuel","sequence":"first","affiliation":[{"name":"Department of Paediatric Surgery, Great Ormond Street Hospital for Children , London,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L","family":"Spitz","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery, Great Ormond Street Hospital for Children , London,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1995,6,1]]},"reference":[{"key":"2024092816200853300_bib1","first-page":"641","article-title":"Du noevus variqueux osteohypertrophiques","volume":"185","author":"Klippel","year":"1900","journal-title":"Archives of General Medicine (Paris)"},{"key":"2024092816200853300_bib2","first-page":"289","article-title":"Congenital mixed vascular deformities of the limbs and their associated lesions","volume":"14","author":"Young","year":"1978","journal-title":"Birth Defects"},{"key":"2024092816200853300_bib3","doi-asserted-by":"crossref","first-page":"624","DOI":"10.1097\/00000658-198511000-00015","article-title":"The etiology of the Klippel-Trenaunay syndrome","volume":"202","author":"Baskerville","year":"1985","journal-title":"Ann Surg"},{"key":"2024092816200853300_bib4","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1097\/00000658-198503000-00020","article-title":"Klippel and Trenaunay's syndrome. 768 operated cases","volume":"201","author":"Servelle","year":"1985","journal-title":"Ann Surg"},{"key":"2024092816200853300_bib5","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1097\/00000658-196508000-00023","article-title":"The Klippel-Trenaunay syndrome: varicosity hypertrophy and hemangioma with no arteriovenous fistula","volume":"162","author":"Lindenauer","year":"1965","journal-title":"Ann Surg"},{"key":"2024092816200853300_bib6","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1002\/bjs.1800720331","article-title":"The Klippel-Trenaunay syndrome: clinical, radiological and haemodynamic features and management","volume":"72","author":"Baskerville","year":"1985","journal-title":"Br J Surg"},{"key":"2024092816200853300_bib7","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1001\/archsurg.1976.01360220119020","article-title":"Congenital arteriovenous anomalies of the limbs","volume":"111","author":"Szilagyi","year":"1976","journal-title":"Arch Surg"},{"key":"2024092816200853300_bib8","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1001\/archpedi.1940.01990160135009","article-title":"Capillary hemangioma with extensive purpura: report of a case","volume":"59","author":"Kasabach","year":"1940","journal-title":"Am J Dis Child"},{"key":"2024092816200853300_bib9","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1016\/S0022-3476(75)80634-2","article-title":"Macrocephaly in association with unusual cutaneous angiomatosis","volume":"87","author":"Stephan","year":"1975","journal-title":"J Pediatr"},{"key":"2024092816200853300_bib10","first-page":"231","article-title":"Angioma-formation in connection with hypertrophy of limbs and hemi-hypertrophy","volume":"19","author":"Parkes-Weber","year":"1907","journal-title":"Br J Dermatol"},{"key":"2024092816200853300_bib11","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1111\/j.1365-2133.1992.tb00107.x","article-title":"Genetic aspects of the Klippel-Trenaunay syndrome","volume":"126","author":"Aelvoet","year":"1992","journal-title":"Br J Dermatol"},{"key":"2024092816200853300_bib12","first-page":"488","volume-title":"The Phakomatoses. 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